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Enfermedad relacionada con IgG4: vasculitis cutánea que se presenta como múltiples nódulos subcutáneos
Gregory S Olson1, Ritika Walia1, Allison Cargnel2
1Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA.
The American Journal of dermatopathology
|December 23, 2025
Resumen
Un estudio de caso revela que la enfermedad relacionada con IgG4 se presenta como nódulos subcutáneos con flebitis obliterante. Este hallazgo amplía la comprensión de las manifestaciones de la enfermedad relacionada con IgG4, enfatizando la participación vascular.
Área de la Ciencia:
- Patología
- Inmunología
- Dermatología
Sus antecedentes:
- La enfermedad relacionada con IgG4 (IgG4-RD) es una afección fibroinflamatoria.
- Las manifestaciones cutáneas de la IgG4-RD son diversas pero a menudo subreconocidas.
- La flebitis obliterante es una característica conocida de la IgG4-RD, pero su presentación en nódulos subcutáneos es menos común.
Objetivo del estudio:
- Informar un caso único de enfermedad relacionada con IgG4 que se presenta como nódulos subcutáneos.
- Destacar las características histopatológicas y los criterios de diagnóstico para IgG4-RD en este contexto.
- Enfatizar la importancia de considerar la participación vascular en la IgG4-RD cutánea.
Principales métodos:
- Examen histopatológico de nódulos subcutáneos extirpados.
- Inmunohistoquímica para células plasmáticas positivas para IgG4 y relación IgG:IgG4.
- Exclusión de otras etiologías potenciales, incluidas neoplasias e infecciones.
Principales resultados:
- La histopatología reveló una lesión intravascular bien delimitada con infiltrado linfoplasmocítico, histiocitos y eosinófilos.
- La inmunohistoquímica confirmó >200 células plasmáticas policlonales de IgG4/campo de alta potencia y una relación IgG4:IgG >0.5.
- Se estableció el diagnóstico de enfermedad relacionada con IgG4 con flebitis obliterante de una vena subcutánea de tamaño mediano.
Conclusiones:
- Este caso amplía la clasificación de la enfermedad cutánea relacionada con IgG4.
- Subraya la importancia de reconocer las manifestaciones vasculares en la IgG4-RD.
- Una mayor concienciación sobre estas presentaciones es crucial para el diagnóstico y el manejo oportunos.
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